Right Aortic Arch with Isolation of the Left Subclavian Artery and Bilateral Patent Ductus Arterioses

Of the right aortic arch anomalies, a right arch with isolation of the left subclavian artery is the least common. In a neonate who had a right arch with isolation of the left subclavian artery and bilateral patent ductus arterioses, pulmonary artery steal phenomenon developed after ligation of the right ductus. We performed division of the left ductus and reimplantation of the left subclavian artery into the left common carotid artery.     

Congenital Pulmonary Steal Phenomenon Associated with Tetralogy of Fallot, Right Aortic Arch, and Isolation of the Left Subclavian Artery

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).     

Partial Anomalous Left Pulmonary Artery: New Evidence on the Development of the Pulmonary Artery Sling

Clinical and angiographic data of a child with a unique form of partial anomalous left pulmonary artery are reported. Because the anomalous pulmonary artery does not run posterior to the trachea, this malformation is not associated with airway obstruction, as are all other forms of anomalous left pulmonary artery described to date. This case strengthens our understanding of the development of the pulmonary artery sling.     

Dobutamine Stress Echocardiography in Anomalous Left Coronary Artery

The evaluation of left ventricular function with dobutamine stress echocardiography is described for the first time in a patient with anomalous left coronary artery from the pulmonary trunk during the preoperative and postoperative periods. This method demonstrated signs of myocardial ischemia that were not seen on the resting echocardiogram during the preoperative period and ventricular function recovery after surgical intervention.     

Electrocardiographic and Echocardiographic Features That Distinguish Anomalous Origin of the Left Coronary Artery from Pulmonary Artery from Idiopathic Dilated Cardiomyopathy

Many authors have reported noninvasive means of diagnosing anomalous left coronary artery from pulmonary artery (ALCAPA) and differentiating ALCAPA from idiopathic dilated cardiomyopathy (DCM). Systematic evaluation using these noninvasive diagnostic modalities is not available. To distinguish between ALCAPA and DCM using a systematic approach, we examined 23 patients with ALCAPA (age 1 month to 23 years, median 7 months) and 23 patients with DCM (age 5 days to 16 years, median 6.6 months). Standard 12-lead electrocardiograms (ECG) and 2-dimensional (2-D) and color Doppler echocardiograms were performed. A logistic regression model was applied using ALCAPA diagnosis as the dependent variable and ECG and echocardiographic findings as independent variables. A scoring system was created to establish the ALCAPA diagnosis based on results from the logistic regression. On the logistic regression, the ECG feature of QT pattern in aVL (Q wave ≥ 3 mm deep with an inverted T wave) and echocardiographic features of right coronary artery diameter to aortic annulus ratio ≥ 0.14, increased papillary muscle echogenicity, and Doppler color flow of LCA from aorta or pulmonary artery were the most significant differentiating features between the ALCAPA and DCM groups. A scoring system was developed using the previous five variables and assigning a score of 1 to each variable (−1 to Doppler color flow of LCA from aorta). The scoring system had sensitivity of 100% and specificity of 91% for ALCAPA diagnosis. Compared with previous reported diagnostic features in differentiating ALCAPA and DCM, the scoring system had a much higher specificity and positive predictive value. In conclusion, we selected the most useful ECG and echocardiographic features to differentiate between ALCAPA and DCM and created a scoring system to aid clinical diagnosis. This scoring system may be useful in evaluating children with acute congestive heart failure.     

Left-Side Pulmonary Vein Obstruction After Arterial Switch Operation in Infants with D-Transposition of the Great Arteries

We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.     

A Case of Anomalous Origin of the Pulmonary Arteries: Right Pulmonary Artery from the Descending Aorta and the Left Pulmonary Artery from the Ascending Aorta

A 3-month-old girl classified as having persistant truncus arteriosus underwent surgical correction of the anomalous origin of the pulmonary arteries; the right pulmonary artery from the descending aorta and the left pulmonary artery from the ascending aorta. The patient died on the fourth postoperative day. The definite diagnosis and choice of surgical strategies should be further examined.     

Origin of the Main Pulmonary Artery from the Left Coronary Artery in Complex Pulmonary Atresia

We describe a 22-year-old woman with a history of unrepaired pulmonary atresia with ventricular septal defect. This woman was interesting in that her main pulmonary artery and right pulmonary artery arose from the left main coronary artery. She developed significant pulmonary hypertension in addition to isolation of the left pulmonary artery following ductal closure, subsequent to which the majority of her pulmonary blood flow was coronary dependent.     

Anomalous Origin of One Pulmonary Artery from the Innominate Artery: A Report of Two Cases

Two children with an anomalous origin of one pulmonary artery from the innominate artery are reported. One was a 15-month-old boy presenting with respiratory distress. He had a right aortic arch and his left pulmonary artery originated from the innominate artery. The other was a 1-month-old girl presenting with congestive heart failure. She had a left aortic arch and her right pulmonary artery originated from the innominate artery. An understanding of the embryological pathogenesis of these anomalies has significant therapeutic implications.     

Anomalous Origin of the Left Coronary Artery in Tetralogy of Fallot Associated with Abnormal Mitral Valve Pathology

The association of anomalous left coronary artery with congenital heart disease is a rare occurrence. Seven cases of anomalous left coronary artery associated with tetralogy of Fallot have been reported in the literature. We report a unique case with severe mitral valve abnormality that precluded standard surgical repair.     

Congenital Subclavian Artery to Subclavian Vein Fistula Presenting with Congestive Heart Failure in an Infant

Contrary to acquired arteriovenous fistulas, the congenital malformations are very rare. Here we report a case of congenital arteriovenous fistula forming a communication between the right subclavian artery to subclavian vein. To our knowledge, there is no similar case in which an infant has been successfully operated on for cardiac failure.     

Double-Outlet Right Ventricle (Tetralogy of Fallot Type) Associated with Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery: Report of Successful Total Repair in a 2-Month-Old Infant

The case reported herein demonstrates the rare association of double-outlet right ventricle (tetralogy of Fallot type) with anomalous origin of the left coronary artery from the pulmonary artery. It is the first reported successful total surgical repair in an infant.     

Coarctation of the Left Pulmonary Artery: Effects on the Pulmonary Vasculature of Infants

At autopsy, two infants had unsuspected coarctation of the left pulmonary artery (CoLPA), which was produced by an extension of ductal tissue into the wall of the left pulmonary artery. The first case, a 4-month-old girl, also had a ventricular septal defect and an anomalous branching pattern of the innominate arterial trunk. Pulmonary arterial hypertensive changes were noted in the right lung. In contrast, the left lung showed thin-walled pulmonary arteries. The second case, a term female newborn, had exhibited severe unexplained respiratory distress since birth. Histologic sections of the right lung showed dilated pulmonary arteries with thinned media, whereas the left lung showed a persistent fetal arterial pattern. It is believed that the peripheral pulmonary arterial changes are age-dependent and associated with asymmetric blood flow between the right and left pulmonary arteries. CoLPA is a rare pulmonary artery defect, and early diagnosis of this abnormality is important.     

Unusual Longevity Without Surgical Intervention in Complete Transposition of the Great Arteries

A case of unusual longevity to the age of 58 years is reported for a female patient with complete transposition of the great arteries. The association with a wide atrial septal defect with intact interventricular septum may have contributed to the long survival without surgery. Factors determining intercirculatory mixing and systemic oxygen saturation may be the high pulmonary flow, the location of the anatomic communication, sufficient hemoglobin concentration to allow an adequate level of systemic resistance and recirculated systemic flow, and the belated development of pulmonary vascular disease.     

Down Syndrome as a Factor Influencing Hemodynamic Response to Pulmonary Artery Banding

The hemodynamic response to pulmonary artery banding (PAB) in relation to the preoperative pulmonary/systemic vascular resistance (R_p/R_s) ratio and to the timing of surgery, with special regard to Down syndrome, was investigated in 56 nonconsecutive pediatric patients aged 3 days to 6 months (mean 2.5 months) with simple and complex congenital shunt-related cardiac malformations. Among the non-Down patient group (39 patients; mean age 6.9 weeks) there was a good hemodynamic response in all but three cases, irrespective of the preoperative R_p/R_s ratio; these three poor responders had preoperatively normal or nearly normal R_p/R_s ratios (R_p/R_s < 0.3) and were affected postoperatively by lung complications. In the Down patient group (17 patients; mean age 8.2 weeks) the mean preoperative as well as the mean postoperative R_p/R_s ratio was higher than in the non-Down patient group (preoperative R_p/R_s 0.49 versus 0.32; postoperative R_p/R_s 0.31 versus 0.18). There was a good hemodynamic response in all five patients with Down syndrome who had preoperative normal or nearly normal pulmonary vascular resistance ratios (R_p/R_s < 0.3). Among 12 patients with Down syndrome and preoperative increased resistance ratios (R_p/R_s > 0.3) PAB did not cause a reduction in pulmonary vascular resistance (PVR) in five patients (postoperative R_p/R_s 0.49–1.00), all operated on at more than 6 weeks of age. PAB resulted in effective reduction of postoperative R_p/R_s ratios (range 0.10–0.27) in seven patients, six of them younger and one older than 6 weeks at the time of the banding procedure. In conclusion, patients with Down syndrome and shunt-related cardiac malformations (predominantly total atrioventricular canal cases) in general have higher pre- and postoperative R_p/R_s ratios than non-Down children and also have a higher potential for developing pulmonary vascular obstructive disease despite hemodynamically effective PAB. Especially in children with Down syndrome and pathologically high resistance ratios, PAB, if indicated, should be performed as early as possible.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Ventricular Septal Defect and Mitral Stenosis

We report a case of a child with ventricular septal defect, mitral stenosis, and patent ductus arteriosus, who was also found to have anomalous origin of the left coronary artery from the pulmonary artery. Preoperative diagnosis allowed successful surgical correction.     

Association of Common Origin of the Carotid Arteries with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Common origin of the carotid arteries (COCA) is a common pattern of aortic arch vessels and is the single most common cause of tracheobronchial compression by a congenital cardiovascular anomaly. By no means all affected patients are symptomatic. Symptoms may range from recurrent pulmonary infections and “noisy respiration” to stridor and apneic spells. In our study of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) we found a highly significant association of COCA with ALCAPA (85%), although no patient with ALCAPA in this study had evidence of tracheal stenosis documented in the hospital chart. As COCA is easily correctable, we suggest consideration of COCA during evaluation and surgery of patients with ALCAPA so that, if the patient also has symptoms possibly related to COCA, the artery can be suspended from the posterior wall of the sternum.     

Transposition of the Great Arteries, Pulmonary Atresia, and Multiple Ventricular Septal Defects Associated with Multiple Cardiac Rhabdomyomas in a Case of Tuberous Sclerosis

Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures.     

Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection in an Adult

Adult survival in patients with uncorrected obstructed infracardiac total anomalous pulmonary venous drainage has not been reported. A 17-year-old man who presented to us with features of severe pulmonary arterial hypertension was diagnosed to have obstructed pulmonary venous drainage to the hepatic vein. Presence of a large ventricular septal defect in this patient may have contributed favorably to survival.     

Anomalous Origin of Coronary Artery in Association with Aorticopulmonary Window

Aorticopulmonary window associated with anomalous origin of coronary arteries is rare; only 12 cases have been reported previously. Origin of coronary arteries from the communicating bridge is rare, having occurred in only five of these cases. We describe two additional cases of this entity, in one of which the right coronary artery arose from the window proper. We also review the previously reported cases.